Cardiac Sarcoidosis Presenting as Sustained Ventricular Tachycardia.

Introduction: Sarcoidosis has many possible clinical presentations since it can affect any organ, most commonly the lungs. The hallmark of the disease consists of the formation of non-necrotising granulomas. Pathogenesis is thought to rely on the interplay of genetic, environmental and epigenetic factors. This case highlights the importance of a thorough clinical history and physical examination, and the correlation with imaging findings in the diagnostic work-up of the non-ischaemic cardiomyopathy. Case description: A 57-year-old woman was admitted due to the sudden onset of malaise, dizziness, and chest discomfort. Sustained monomorphic ventricular tachycardia was evidenced and the patient rapidly evolved with haemodynamic instability; she underwent successful electrical cardioversion. The electrocardiogram afterwards showed a high-risk electrocardiographic pattern. Invasive coronary angiography excluded obstructive epicardial coronary lesions. Physical examination revealed skin lesions on the lower limbs which raised suspicion for erythema nodosum and therefore a biopsy was performed. Transthoracic echocardiography and cardiac magnetic resonance imaging revealed features consistent with an inflammatory cardiomyopathy, and an implantable cardioverter-defibrillator was placed. The histologic examination of the cutaneous lesions showed a non-necrotising granulomatous inflammatory process. Radionuclide imaging was inconclusive. The patient underwent an endomyocardial biopsy, which confirmed the diagnosis of systemic sarcoidosis with cardiac involvement. Conclusions: Systemic sarcoidosis with cardiac involvement is a challenging diagnosis. The role of imaging techniques such as transthoracic echocardiography, cardiac magnetic resonance imaging and radionuclide imaging is essential in raising suspicion and diagnosing this pathology. Endomyocardial biopsy is the 'gold standard' for its diagnosis; however, it has a low diagnostic yield. LEARNING POINTS: Systemic sarcoidosis with cardiac involvement is a challenging diagnosis as it may present in many different ways.The case presented highlights the importance of a thorough clinical history and physical examination, and the correlation with imaging findings.Imaging techniques such as transthoracic echocardiogram, cardiac magnetic resonance and radionuclide imaging are essential in raising suspicion and diagnosing cardiac sarcoidosis.

Portuguese Heart Failure Prevalence Observational Study (PORTHOS) rationale and design - A population-based study.

INTRODUCTION AND OBJECTIVES: Current epidemiological data on heart failure (HF) in Portugal derives from studies conducted two decades ago. The main aim of this study is to determine HF prevalence in the Portuguese population. Using current standards, this manuscript aims to describe the methodology and research protocol applied. METHODS: The Portuguese Heart Failure Prevalence Observational Study (PORTHOS) is a large, three-stage, population-based, nationwide, cross-sectional study. Community-dwelling citizens aged 50 years and older will be randomly selected via stratified multistage sampling. Eligible participants will be invited to attend a screening visit at a mobile clinic for HF symptom assessment, anthropomorphic assessment, N-terminal pro-B-type natriuretic peptide (NT-proBNP) testing, one-lead electrocardiogram (ECG) and a sociodemographic and health-related quality of life questionnaire (EQ-5D). All subjects with NT-proBNP ≥125 pg/mL or with a prior history of HF will undergo a diagnostic confirmatory assessment at the mobile clinic composed of a 12-lead ECG, comprehensive echocardiography, HF questionnaire (KCCQ) and blood sampling. To validate the screening procedure, a control group will undergo the same diagnostic assessment. Echocardiography results will be centrally validated, and HF diagnosis will be established according to the European Society of Cardiology HF guidelines. A random subsample of patients with an equivocal HF with preserved ejection fraction diagnosis based on the application of the Heart Failure Association preserved ejection fraction diagnostic algorithm will be invited to undergo an exercise echocardiography. CONCLUSIONS: Through the application of current standards, appropriate methodologies, and a strong research protocol, the PORTHOS study will determine the prevalence of HF in mainland Portugal and enable a comprehensive characterization of HF patients, leading to a better understanding of their clinical profile and health-related quality of life.

conTemporary reflectiOns regarding heart failure manaGEmenT - How to ovERcome the PorTuguese barriers (TOGETHER-PT).

INTRODUCTION AND OBJECTIVES: Heart failure (HF) is a complex clinical syndrome that is a significant burden in hospitalisations, morbidity, and mortality. Although a significant effort has been made to better understand its consequences and current barriers in its management, there are still several gaps to address. The present work aimed to identify the views of a multidisciplinary group of health care professionals on HF awareness and literacy, diagnosis, treatment and organization of care, identifying current challenges and providing insights into the future. METHODS: A steering committee was established, including members of the Heart Failure Study Group of the Portuguese Society of Cardiology (GEIC-SPC), the Heart Failure Study Group of the Portuguese Society of Internal Medicine (NEIC-SPMI) and the Cardiovascular Study Group (GEsDCard) of the Portuguese Association of General and Family Medicine (APMGF). This steering committee produced a 16-statement questionnaire regarding different HF domains that was answered to by a diversified group of 152 cardiologists, internists, general practitioners, and nurses with an interest or dedicated to HF using a five-level Likert scale. Full agreement was defined as ≥80% of level 5 (fully agree) responses. RESULTS: Globally, consensus was achieved in all but one of the 16 statements. Full agreement was registered in seven statements, namely 3 of 4 statements for patient education and HF awareness and 2 in 4 statements of both HF diagnosis and healthcare organization, with proportions of fully agree responses ranging from 82.9% to 96.7%. None of the HF treatment statements registered full agreement but 3 of 4 achieved ≥80% of level 4 (agree) responses. CONCLUSION: This document aims to be a call-to-action to improve HF patients' quality of life and prognosis, by promoting a change in HF care in Portugal.

SGLT-2 inhibitors: A step forward in the treatment of heart failure with reduced ejection fraction.

Heart failure (HF) is a major health problem with a significant impact on morbidity, mortality, quality of life and healthcare costs. Despite the positive impact of disease-modifying therapies developed over the last four decades, HF mortality and hospitalization remain high. We aim at reviewing the evidence supporting the use of sodium-glucose co-transporter-2 (SGLT-2) inhibitors, as a novel strategy for HF with reduced ejection fraction (HFrEF) treatment. The consistent observation of a reduction in HF hospitalizations in type-2 diabetes cardiovascular safety trials EMPA-REG OUTCOME, CANVAS, DECLARE-TIMI 58 and VERTIS raised the hypothesis that SGLT-2 inhibitors could have an impact in HF treatment. This hypothesis was first confirmed in 2019 with the DAPA-HF publication showing that dapagliflozin on top of optimized HFrEF therapy, reduced HF-hospitalizations and cardiovascular mortality. This was reinforced by the EMPEROR-Reduced publication in 2020 showing that empagliflozin on top of optimized HFrEF therapy, reduced HF-hospitalizations. Both studies established SGLT-2 inhibitors as a fourth pillar of HFrEF prognosis-modifying therapy, in addition to the gold standard triple neurohormonal modulation/blockade.

Obstructive sleep apnea syndrome: An important piece in the puzzle of cardiovascular risk factors.

The obstructive sleep apnea syndrome (OSA) is a clinical entity characterized by recurring episodes of apnea and/or hypopnea during sleep, due to a total or partial collapse, respectively, of the upper airway. This collapse originates a set of pathophysiological changes that determine the appearance of several cardiovascular complications. OSA contributes for the development of hypertension, heart failure, arrhythmias and coronary heart disease. Nowadays it is recognized to be an important public health problem, taking into account not just its repercussions but also its prevalence, since the main risk factor for the disease is obesity, a growing problem worldwide, both in developed and developing countries. The present review summarizes the current knowledge about OSA, as regards its definition, pathophysiology, clinical manifestations, diagnosis, cardiovascular effects and treatment.

Effusive-constrictive pericarditis as the manifestation of an unexpected diagnosis.

Constrictive pericarditis is a clinical condition characterized by the appearance of signs and symptoms of right heart failure due to loss of pericardial compliance. Cardiac surgery is now one of the most frequent causes in developed countries, while tuberculosis remains the most prevalent cause in developing countries. Malignancy is a rare cause but usually has a poor prognosis. The diagnosis of constrictive pericarditis remains a clinical challenge and requires a combination of noninvasive diagnostic methods (echocardiography, cardiac magnetic resonance and computed tomography); in some cases, cardiac catheterization is needed to confirm the diagnosis. The authors present the case of a 51-year-old man, hospitalized due to cardiac tamponade. Diagnostic investigation was suggestive of tuberculous etiology. Despite directed medical therapy, the patient developed effusive-constrictive physiology. He underwent pericardiectomy and anatomopathologic study suggested a neoplastic etiology. The patient died in the postoperative period from biventricular failure.

A case of rheumatic valvular heart disease and autoimmune gastritis.

We present a case of a 50-year-old female patient with a history of depressive disorder and anaemia (attributed to menorrhagias). She was admitted to the cardiology department with symptoms of fatigue on moderate exertion for several months, with worsening in the month before hospitalisation. Echocardiography revealed a severe mitral stenosis of rheumatic aetiology. Laboratory tests showed microcytic and hypochromic anaemia, reduced iron stores and vitamin B12 levels, and positive serum antiparietal cells autoantibodies. Endoscopy showed focal areas of erythema in the stomach, corresponding histologically to chronic atrophic gastritis. In this context, two distinct clinical entities were diagnosed in the same patient: severe rheumatic mitral stenosis and autoimmune gastritis. The patient was started on vitamin B12 and iron supplementation and underwent surgical correction of the valvular disease. There was symptomatic improvement in her signs of fatigue.

Retinal artery embolization complicating Libman-Sacks endocarditis in a systemic lupus erythematosus patient.

Libman-Sacks endocarditis (LSE) is the most characteristic cardiac manifestation of systemic lupus erythematosus (SLE). It is usually clinically silent but heart failure due to valvular dysfunction, secondary infective endocarditis and embolic phenomena can complicate valvular abnormalities. We present a patient with SLE and blindness due to right central retinal artery occlusion. Echocardiographic examination revealed a verrucous vegetation on the posterior mitral valve leaflet consistent with LSE. Anticoagulation therapy was started. Echocardiographic regression of the vegetation was observed and there has been no recurrence of thromboembolic events to date.

[Giant left atrial myxoma presenting as acute myocardial infarction]. / Enfarte agudo do miocárdio como forma de apresentação de mixoma gigante da aurícula esquerda.

Myxomas are the most common type of benign cardiac tumor. The most frequent clinical presentations are symptoms resulting from atrioventricular valve obstruction or systemic embolization. Coronary embolization is a rare, although real and potentially fatal, complication of cardiac myxomas. We present a case report and review of the literature on this disease association. A 57-year-old woman was admitted to our coronary care unit with a diagnosis of non-ST elevation acute myocardial infarction. Transthoracic echocardiography showed a large left atrial mass attached to the interatrial septum, coral-like and with a friable appearance, suggestive of myxoma. Coronary angiography revealed no significant lesions and the patient underwent surgical excision of the mass, which histological study showed to be compatible with myxoma. The postoperative period was uneventful and the patient is doing well, with no recurrence of myxoma.

[Images in cardiology after clinical observation - aortic dissection in Marfan syndrome]. / Imagens em Cardiologia mas depois da clínica em Cardiologia - Dissecção da aorta num doente com Síndrome de Marfan.

INTRODUCTION: Stanford type A aortic dissection is a rare phenomenon with high short-term mortality and clinical manifestations that can make differential diagnosis a lengthy process requiring several diagnostic examinations. OBJECTIVES: Based on a case report, the aim is to highlight the importance of physical examination in the initial management of these patients and of rapid access to a surgical center. A brief review follows on the diagnosis and treatment of ascending aortic dissection, and its specific nature in Marfan syndrome. CASE REPORT: A 33-year-old man was admitted to the emergency department of a district hospital with chest and back pain associated with vomiting, 20 hours after symptom onset. Initial physical examination revealed an aortic systolic murmur and musculoskeletal morphological abnormalities compatible with Marfan syndrome. Given suspected aortic dissection, a transthoracic echocardiogram was immediately performed, which showed an extensive intimal flap originating at the sinotubular junction. He was transferred to the cardiothoracic surgery department of a referral hospital where he was treated by a Bentall procedure. CONCLUSION: In this case, careful physical examination during initial assessment raised the suspicion that this patient was in a high-risk group for aortic dissection, thus avoiding unnecessary and lengthy exams. This diagnosis requires emergent surgical treatment, and so direct contact in real time between those making in the diagnosis and the surgeon is essential, as well as protocols governing immediate access to a surgical center.

Cardiomyopathy in Churg-Strauss syndrome.

The authors present the case of a 56-year-old man, admitted to the hospital twice in ten days for acute coronary syndrome with normal coronary angiograms. In the second hospitalization, the patient had anginal crises that did not respond to anti-ischemic therapy, associated with cough and wheezing. The echocardiogram revealed worsening left ventricular systolic dysfunction. He had no cardiovascular risk factors but there was a history of bronchial asthma, allergic rhinitis and peripheral neuropathy of the left upper limb with paresthesias. Laboratory studies showed eosinophilia, detected in previous blood tests, although more marked than before. Chest X-rays showed non-fixed pulmonary infiltrates and bronchoalveolar lavage revealed increased lymphocytes and eosinophils, suggesting Churg-Strauss syndrome with the probable cardiac manifestation of coronary vasospasm. A cardiac MRI was also performed but was inconclusive due to the patient's intolerance of the exam.

Transesophageal echocardiography in an emergency setting. A district general hospital experience.

Transesophageal echocardiography is an extremely useful technique for the study of various cardiovascular pathologies. In the particular setting of emergency, it is of great value for prompt diagnosis and appropriate therapy. It was our aim to evaluate, in our hospital, the benefits obtained by the use of transesophageal echocardiography in an emergency setting. We retrospectively studied patients who underwent transesophageal echocardiography (TEE) in an emergency setting, from June 1997 to December 2002, evaluating demographic characteristics, indication to perform TEE, benefit obtained (diagnosis or exclusion of initial diagnosis), and technique-related complications. There were 97 transesophageal echocardiograms performed in an emergency setting in the period under consideration, accounting for 19.3% of the total number of exams. Fifty-two patients (53.6%) were male, mean age 63.9 +/- 12.7. Nineteen patients (19.6%) were on assisted ventilation. The indications to perform TEE were: possible massive or submassive pulmonary thromboembolism in 32 patients (33.0%); suspected aortic dissection in 19 (19.6%); shock with inconclusive transthoracic echocardiogram in ten (10.3%); possible endocarditis in eight (8.2%); possible prosthetic valve dysfunction in seven (7.2%); intracardiac mass in six (6.2%); search for cardiac source of embolism in five (5.2%); possible mechanical complication of acute myocardial infarction in four (4.1%); pre-electrical cardioversion study in four patients with atrial fibrillation (4.1%); and suspected congenital heart disease in two (2.1%). TEE examination yielded additional information and helped in the therapeutic decision in 88 patients (90.7%), leading to a diagnosis in 49 (50.6%), which was different from the initial diagnostic hypothesis in four, and exclusion of the suspected diagnosis in 39 (40.1%). There was only one minor complication (1.0%) and no TEE-related mortality. We concluded that transesophageal echocardiography is an extremely useful and safe cardiovascular diagnostic technique in an emergency setting in a district general hospital, enabling a diagnosis to be reached or excluded in almost all patients, which is essential for implementing appropriate therapy.

Pulmonary embolism associated with a large tricuspid-related mass.

The case of a 43-year-old man with diabetes and alcoholism admitted to the emergency room with shock, fever, pleuritic chest pain and systemic symptoms is presented. Laboratory tests revealed anemia, leukocytosis, thrombocytopenia, high sedimentation rate and D-dimers, hypoxemia and hypocapnea. He also had sinus tachycardia, rSR' in V1 and an opacity on the periphery of the right pulmonary field. Blood and urine cultures were negative, as were serological markers. The echocardiogram showed a large mass adhering to the tricuspid valve, suggestive of myxoma. The patient underwent surgery, and anatomopathological examination of the mass showed it to be a bacterial vegetation, with no agent isolated. It is pointed out that differential diagnosis is difficult between a myxoma with systemic symptomatology associated with a possible pulmonary embolism, and tricuspid endocarditis with negative blood culture associated with a septic pulmonary embolism, which turned out to be the diagnosis in this patient.